Benign Soft Tissue

Tumors
Lipoma, Hemangioma, Fibromatosis
Myxoma, Schwannoma, GCT of Tendon Sheath
Pigmented Villonodular Synovitis (PVNS

James C. Wittig, MD
Orthopedic Oncologist
Sarcoma Surgeon
www.TumorSurgery.org

Lipoma
Definition: Benign tumor composed of mature
adipocytes with uniform nuclei identical to the
cells of normal adult fat.
Benign/Mature adipocytes have cytoplasmic lipid
in a single large vacuole that pushes the nucleus to
the periphery of the cell and compresses it into a
thin crescent.
Lipoblasts: Malignant or Immature adipocytes.
Larger peripheral or central nucleus indented by one or
more fat vacuoles so that spikes of chromatin project
between fatty vacuoles. Usually smaller and have
smaller vacuoles than mature lipocytes

Lipoma
Types of Lipomas:

Lipoma
Fibrolipoma
Angiolipoma
Spindle Cell/Pleomorphic Lipoma
Myxolipoma
Lipoblastoma
Myolipoma
Hibernoma
Chondroid Lipoma

Lipoma

Most common soft tissue tumor

Adults
Location:

Subcutaneous: Most Common

Back, shoulder, neck, abdomen, proximal extremities
Uncommon in hands, feet, distal extremities
Rarely recur after excision
No sex predilection: Males=Females
Deep
Intramuscular: develops within skeletal muscle
Usually affects adults but occasionally found in children
Trunk, large muscles of arm, thigh, shoulder
Males affected greater than females
Up to 20% recurrence after excision especially if infiltrative

Lipoma
Clinical Presentation:
Slowly growing, asymptomatic, painless mass
Some patients may have multiple lipomas

Radiology: Lipoma
Radiology: Follows that of subcutaneous fat
Xray: Radiolucent soft tissue mass; Calcification may be present
particularly in areas of fatty necrosis
CT Scan: Well defined, homogeneous
No enhancement following contrast administration
Tissue Attenuation Coefficient is Low (-65 to -120 HU)
MRI: Follows the signal characteristics of subcutaneous fat on all
sequences
T1: High Signal
T2: Intermediate Signal; Low Signal with Fat Suppression
Minimal or No enhancement with gadolinium

MRI T1: Intramuscular Lipoma of Thigh

High Signal on T1

Signal Same as Subcutaneous Fat

Minimal Stranding within Tumor

MRI T2 Fat Suppressed Image

Fat in Lesion Suppressed Same as Subcutaneous Fat

MRI T1: Lipoma of Posterior Thigh

MRI T2 (not Fat Suppressed)

Fat is Intermediate Signal on T2
Low Signal on Fat Suppressed

MRI T1: Lipoma Right Shoulder

Fibrolipoma: Notice Stranding
Stranding within Tumor
indicates
Collagenous/Fibrous
Septae
Can Also Occur with Low
Grade
Liposarcomas/Atypical
Lipomas

MRI T1: Lipoma of Shoulder

MRI T2 Fat Suppressed

MRI T1: Large Lipoma Left Thigh

with Small Area of Fat Necrosis

Large Lipoma
Area of Fat
Necrosis

Pathology: Lipoma
Gross Pathology:

Soft yellow fatty mass indistinguishable from normal fat

Lobular growth in some lipomas
May have fibrous component reflected as white tissue
Muscle fibers may be present with intramuscular lipomas

Gross Pathology Lipoma

Yellow fat
Area of necrosis (arrow)

Pathology: Lipoma
Microscopic Pathology:
Mature fat cells with small, uniform, eccentric nuclei
Nucleus compressed against the periphery of the cell membrane
by a fat vacuole

No mitotic figures
Can have areas that undergo fat necrosis
Muscle fibers interspersed amongst mature adipocytes
(intramuscular lipomas)
Other components
Fibrous tissue
Myxoid tissue
Blood vessels

Pathology Microscopic Lipoma

Uniform Cells
Peripheral
compressed nuclei
barely discernible
Mature adipocytes
No mitoses

Pathology Microscopic Lipoma

Peripheral
Compressed
Nuclei

Compressed
nuclei are
barely
discernible

Differential Diagnosis
Lipoma vs atypical lipomatous tumor (well
differentiated liposarcoma)
Both may look similar on MRI
Well differentiated liposarcoma:
Larger nuclei
Lipoblasts
Considerable variation in fat cell size

Differential Diagnosis
Lipomas do not occur in the retroperitoneum.
Retroperitoneal fatty tumors are capable of
recurring and dedifferentiating even if they look
histologically bland similar to a lipoma
Retroperitoneal fatty tumors should be
considered malignant

Treatment and Prognosis

Lipomas: Benign and do not metastasize
Not treated with any forms of chemotherapy nor
radiation.

Observation for small asymptomatic lipomas

Surgery: Marginal excision for symptomatic,
large or deep lesions
Local recurrence:
Risk depends on size and location of lipoma
Rarely occurs for subcutaneous tumors
Intramuscular tumors--up to 20% local recurrence
rate.

Hemangioma
Defintion: Benign proliferation of mature vessels.
Composed of capillaries, veins or a combination of both
Vessels vary in size and shape.
Broad variety of hemangiomas with varying clinical
presentations and biological activity.

Sites:

Skin
Subcutaneous
Intramuscular
Intraarticular/Synovial
Bone

Intramuscular Hemangioma
Often larger than cutaneous hemangiomas
More often symptomatic
Recur more often than cutaneous hemangiomas

Intramuscular Hemangioma
Composed of morphologically benign vascular
channels occurring within skeletal muscle
Almost always associated with varying amounts
of fatty tissue
Blood vessels/vascular channels are often mixed
type of venous and capillary.

Intramuscular Hemangioma
Adolescent and Adult most frequently affected
Male=Female
Sites:

Lower Limb most common

Head and Neck
Upper Limb
Trunk
Rare: Retroperitoneum and Mediastinum

Intramuscular Hemangioma
Clinical:

Slowly enlarging often longstanding mass

Painful
Pain worse after exercise
Changes size according to position of limb

Radiology:
Intramuscular Hemangioma
Xray: Usually Normal
Phleboliths or stromal bone formation (30-50%)
Pressure erosion of adjacent bone or extension into
bone (channel like radiolucencies)

CT: Poorly defined lesion attenuation similar to

muscle
Marked contrast enhacement of serpentine vascular
channels
Subtle phleboliths
Lesions adjacent to bone rarely stimulate a subtle
periosteal reaction

MRI: Hemangioma

Hetero. poorly marginated mass on T1

High SI areas on T1 (fat overgrowth)
Heterogeneous well-defined mass on T2
Vascular channels high T2 SI (slow flow)
Hemorrhage/prominent enhancement
Infiltrate (dont displace or destroy) surrounding
structures

Hemangioma: Phleboliths (arrows)

Hemangioma

Hemangioma

Hemangioma

Hemangioma

Hemangioma

MRI T2 Hemangioma

Hemangioma

Pathology
Intramuscular Hemangioma
Gross Pathology:
Large, poorly demarcated, yellowish (fatty nature)
Vascular and hemorrhagic areas
Focal calcification or ossification

Microscopic Pathology:
Usually mixed vessel type
Lined by normal appearing single layer of epithelial cells
Diffusely infiltrate muscle and entrap muscle cells leading to
degenerative bizarre muscle cells
Prominent adipose component

Hemangioma

Fibromatosis/Desmoplastic
Fibroma
Extra-abdominal Desmoid tumor

Definition:

Benign, nonmetastasizing, infiltrating fibroproliferative

neoplasm
Composed of fibrocytes, fibroblasts, and
myofibroblasts within a collagenous to myxoid stroma.
The cells have uniform, bland nuclear features.
Infiltration of skeletal muscle occurs routinely.

High propensity for local recurrence.

Fibromatosis

15 to 40 most common; described in all ages

Age < 5: Infantile Fibromatosis
Females > Males, slightly

Sites:

Shoulder Girdle/Upper Arm (most common)

Buttock
Trunk
Head and Neck 10%
Hands and Feet: rare
10% are Multicentric and usually involve an anatomic area
although may develop in unrelated areas

Clinical Presentation:
Mass or swelling that may be mildly painful or painless

Radiology: Fibromatosis
Plain Radiographs:
Usually a nonspecific mass
Bone involvement usually pressure
erosion/scalloping if present
No mineralization (rare)
Bone involvement is more common with
recurrences

CT:
Poorly defined margin of mass
Enhance with IV contrast (does not correlate with
lesion vascularity)

Radiology: Fibromatosis
MRI:
Infiltrative growth margin
Tentacles; Invades adjacent tissues; Not ball-like as with other
sarcomas

T1: Intermediate Signal Similar to Muscle

T2: Variable: usually heterogeneous
Low Signal if hypocellular and significant collagen
High signal with hypercellularity, myxoid change /
mucopolysaccharides
Identification of low signal areas on all pulse sequences of dense
collagenous areas
Usually enhance markedly with Gado (10% no enhancement)

MRI T1: Fibromatosis

Intermediate Signal
Not Ball Like
Shoulder Girdle
Area
Serratus Anterior

MRI T2: Fibromatosis

Hyperintense (myxoid
or more cellular)
Subtle low signal
areas in tumor
(fibrous tissue)

MRI T1: Right Buttock Fibromatosis

MRI T2: Fibromatosis Right Buttock

Low to Intermediate
Signal on T2
Significant Fibrous
Component

MRI with Gadolinium: Fibromatosis

Enhances Diffusely
Very Infiltrative
Poor margins

Pathology: Fibromatosis
Gross Pathology:

Most tumors are large (5 to 10 cm and up to 20 cm)

Glitening white fibrous tissue
Myxoid areas may be noted
Infiltrates skeletal muscle at the periphery

Pathology: Fibromatosis
Microscopic Pathology:
Hypocellular to moderately cellular lesion; No Atypia
Elongate, uniform, bland fibroblasts and myofibroblasts
loosely arranged in bundles
Collagenous to myxoid matrix (resembles scar, fascia,
fibrous tissue, tendon)
Often wavy collagen bundles; sometimes large thickened
collagen bundles
Infiltrates skeletal muscle at periphery
Numerous thin walled compressed vessels appear slit-like

Differential Diagnosis
Differentiate from Fibrosarcoma
Difficult to differentiate from Grade 1 Fibrosarcoma

Fibrosarcomas:
More cellular; abnormal chromatin, Mitoses (>5 per
high power field)
Grow more ball-like and less infiltrative although
radiographic appearance can be similar to
fibromatosis

Treatment and Prognosis

Fibromatosis is a benign, nonmetastasizing locally
aggressive neoplasm with a locally destructive
growth pattern and propensity for local recurrence
following resection.
Local destruction of vital structures can lead to
death (ie. growth into thoracic outlet and
mediastinum)

Treatment and Prognosis

Treatment:
Surgery: Wide Excision
Local Recurrence: 20%; Depends on size, location and surgical
margin

Radiation: Controversial
May be considered postoperatively for close or microscopically
positive margins depending on site and multiple patient factors
and if the tumor is recurrent

Chemotherapy: Controversial; Some regimens noted to

shrink some fibromatoses; utilized in selected situations

Myxoma
Definition: Benign hypocellular tumor with
sparse vasculature.
Bland spindle to stellate fibroblasts within an
abundant myxoid/mucinous stroma.
Rare tumor: 1 case per 1 million people
Adults 40 to 60 years old
Females more affected than males
Rare childhood cases
Sites:
Thigh, Gluteus, Upper Arm

Myxoma
Clinical:

Slowly growing mass

Painless
Size usually 5 to 10 cm
Multiple tumors (rare; 5% of patients)
Associated with fibrous dysplasia of underlying bone in
about 5% of cases and in cases of multiple myxomas
(Mazabraud Syndrome)

Radiology: Myxoma
Radiographs:
Normal or a nonspecific soft tissue mass
Mineralization is extremely rare
CT:
Well defined, homogeneous soft tissue mass
No enhancement with CT contrast
MRI: Appear similar to fluid or ganglion cysts
T1: Homogeneous mass; Signal lower than muscle
T2: Markedly high signal
Usually peripheral and septal enhancement with
gadolinium although may see heterogeneous
enhancement

MRI T1: Myxoma of Triceps

MRI T2: Myxoma of Triceps

MRI Myxoma of Triceps

MRI: Myxoma of Thigh

Myxoma of Thigh

Pathology: Myxoma
Gross Pathology:
Round to ovoid, well
circumscribed
Pale mucinous to
gelatinous surface
No Necrosis and
hemorrhage

Myxoma

Myxoma
Microscopic Pathology:
Poorly circumscribed, infiltrative lesion
Merges with surrounding skeletal muscle and fascial tissue
Hypocellular spindle and stellate cells with pale cytoplasm
and dark, hyperchromatic small nuclei
Myxoid/Mucinous Stroma/Matrix that stains pale blue
(Hyaluronic acid)
No nuclear atypia or pleomorphism
Very rare mitoses; No abnormal mitoses
Vimentin positive

Differential Diagnosis
Other Myxoid Neoplasms can appear identical
on MRI

Myxoid MFH
Myxoid Liposarcoma
Myxoid Schwannoma
Myxoid Leiomyosarcoma
Myxoid MPNST
Ganglion Cyst

Treatment and Prognosis

Benign, does not metastasize; grows slowly but
in a nondestructive manner
Treatment: Marginal Excision
Local recurrence<5%
Very low risk of recurrence even when residual
microscopic disease is left behind

Schwannoma/Neurilemmoma
Definition: Benign tumor arising from a peripheral nerve
sheath (epineurium) derived from a schwann cell.
5% of benign soft tissue tumors
Adults; 20 years to 50 years old
Major peripheral nerves in upper and lower extremities,
head and neck region
Clinical: Usually painful, slowly growing mass
Usually <5cm
+Tinels sign
Mobile in transverse plane but no in longitudinal plane

Schwannoma/Neurilemmoma
Arises from the periphery of the nerve,
epineurium
The nerve is displaced eccentrically
Small cutaneous nerves may appear to be
obliterated by the tumor

Radiology: Schwannoma
Plain Radiographs:
Usually normal

CT:
Attenuation on noncontrast CT is lower than muscle

Radiology: Schwannoma
MRI:
Intermuscular mass closely related to a neurovascular
bundle with a distinct margin surrounded by rim of fat
(split fat sign)
Fusiform, elongated cigar shaped mass
Nerve: Tubular structure visualized entering and exiting the
mass (may be difficult to see with small lesions or lesions
of the trunk or retroperitoneum)
Spinal lesions: usually dumbell shaped exiting from and
enlarged neural foramina

Radiology: Schwannoma
MRI:
T1: Isointense to muscle; Variable enhancement with gado
T2: High signal (myxoid areas) with some heterogeneity

Target Sign: Low signal central area (cellular/collagenous

area) and high signal peripheral area (myxoid area)

Pathology: Schwannoma
Microscopic Pathology:
Antoni A Area: Cellular area arranged in short
bundles or interlacing fascicles
Antoni B Area: Less cellular and more myxoid
Ancient schwannomas: cyst formation, calcification,
hemorrhage, fibrosis
S-100 positive staining

MRI T1 and T2: Schwannoma

Target Sign

MRI: Schwannoma
Intermuscular, Neurovascular Region, Target Sign

MRI Schwannoma: Nerve/Tubular

Structure Entering Oblong Mass

MRI: Schwannoma Median Nerve

Target Sign on T2 (arrow)
Hyperintense Area: Myxoid Antoni B
Split Fat Sign on T1

Schwannoma

Median Nerve

Nerve Sheath Opened and Schwannoma Removed; Median Nerve Left Intact

Median Nerve Intact

Schwannoma/Neurilemmoma
Antoni A: Cellular Area

Pathology: Schwannoma
Antoni A: Cellular Area

Pathology: Schwannoma
Antoni B: Myxoid Area

Pathology: Schwannoma
Antoni B: Myxoid Area

Pathology: Schwannoma
Verrucae Bodies

Treatment and Prognosis

Treatment:
Surgical Excision: Marginal Excision; Spare nerve
from excision

Prognosis:
Benign tumor, does not metastasize
Local recurrence rare <1%
Malignant degeneration is very rare

Neurofibromas

5% of all benign soft tissue tumors

90% solitary lesions unrelated to neurofibromatosis
Ages: Young adults 20-30 years old
Sites: usually superficial nerves in the dermis or
subcutaneous tissue
Rarely affect larger nerves
Clinical:
Painless mass less than 5 cm; slowly growing
Infiltrate beyond epineurium into nerve; Not
encapsulated; cannot be separated from the nerve

Neurofibroma
Pathology: interlacing bundles of elongated cells with
wavy, darkly staining nuclei and significant amounts of
collagenization
No Antoni A or Antoni B areas
Solid and fleshy tumors
Rarely removed surgically since they are not painful; Can
not remove without removing nerve
Solitary neurofibromas rarely if ever undergo malignant
change.
Malignant transformation usually occurs in the setting of
neurofibromatosis; Persistently painful lesions usually
indicate malignant transformation to a MPNST which are
usually >5cm

Giant Cell Tumor of Tendon

Sheath
Localized nodular tenosynovitis
Localized or diffuse proliferation of synoviallike cells, giant cells, inflammatory cells and
xanthoma cells along tendon sheaths
3rd-5th decades
Most common benign soft tissue tumor of the
hand
Can erode into bone and destroy it

Giant Cell Tumor of Tendon

Sheath
Hand and wrist
m/c locations (6589%)
Foot and ankle (515%)
Pressure erosions in
15% (esp.
ankles/feet)
Ca+2 uncommon

GCTTS
Isointense to
muscle T1
Heterogeneous on
T2 (Low and High
Signal)
Dark areas on T2:
hemosiderin
May bloom on
gradient echo
(hemosiderin)
May demonstrate
intense
enhancement

Giant Cell Tumor of Tendon

Sheath

GCTTS Hand

GCTTS of Hand

MRI T1: Giant Cell Tumor of

Tendon Sheath

MRI T2: Giant Cell Tumor of

Tendon Sheath of Hand

Pathology: Giant Cell Tumor of

Tendon Sheath
Localized or diffuse proliferation of synoviallike cells, giant cells, inflammatory cells and
xanthoma cells along tendon sheaths
Same histology as PVNS (Pigmented
Villonodular Synovitis)

Pathology: Giant Cell Tumor of

Tendon Sheath

Pathology: Giant Cell Tumor of

Tendon Sheath

Pathology: Giant Cell Tumor of

Tendon Sheath

Pathology: Giant Cell Tumor of Tendon Sheath

Foamy Histiocytes (arrow)

Pigmented Villonodular Synovitis

Large joints (80% in knee)
Joint effusion/pain
Diffuse synovial process
with pathology similar to
GCTTS
Synovial hyperplasia with
multinucleated GC
Intra- and extracellular
hemosiderin

PVNS Imaging
Effusion or mass may be
seen on radiographs
Erosions (50%) m/c in
smaller tighter joints,
especially in hip (93%)
and shoulder (75%)
Erosions are
geographic/lytic +
sclerotic rim
Joint space usually
preserved
Ca+2 very rare
Possible increased
attenuation on CT
(hemosiderin)

PVNS Imaging
Heterogeneous synovial
mass extending away
from joint on MR
SI < muscle on T1 and
T2
Scattered areas of high
SI on T2 possible
Cystic lesions
uncommon (10%)
Blooming on gradient
images (hemosiderin)

Thank You!